ArticleJournal of neuro-oncology2019
Partnership for defining the impact of 12 selected rare CNS tumors: a report from the CBTRUS and the NCI-CONNECT.
Article in Journal of neuro-oncology, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 16 papers.
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Who cites it
16 citing papers in PubMed.
- A phase II study of dose-dense temozolomide and lapatinib for recurrent low-grade and anaplastic supratentorial, infratentorial, and spinal cord ependymoma.Neuro-oncology · 2021Trial
- 8C7: A Fully Human Anti-PTGFRN Monoclonal Antibody-Drug Conjugate Inhibiting Tumour Growth of Mesothelioma and Paediatric Medulloblastoma Cell Lines.Journal of cellular and molecular medicine · 2025Article
- Article
- Virtual multi-institutional tumor board: a strategy for personalized diagnoses and management of rare CNS tumors.Journal of neuro-oncology · 2024Article
- An adult with recurrent atypical teratoid rhabdoid tumor of the spine.CNS oncology · 2024Article
- Capturing evolving definitions of 12 select rare CNS tumors: a timely report from CBTRUS and NCI-CONNECT.Journal of neuro-oncology · 2023Article
- The Role of Immunotherapy in the Treatment of Rare Central Nervous System Tumors.Current oncology (Toronto, Ont.) · 2023Review
- Comprehensive profiling of myxopapillary ependymomas identifies a distinct molecular subtype with relapsing disease.Neuro-oncology · 2022Article
- Multimodal targeting of glioma with functionalized nanoparticles.Cancer cell international · 2022Review
- Chasing a rarity: a retrospective single-center evaluation of prognostic factors in primary gliosarcoma.Strahlentherapie und Onkologie : Organ der Deutschen Rontgengesellschaft ... [et al] · 2022Article
- Article
- NCI-CONNECT rare CNS tumor initiative: current progress and anticipated future impact.Neuro-oncology advancesArticle
- Clinical characterization of adult medulloblastoma and the effect of first-line therapies on outcome; The MD Anderson Cancer Center experience.Neuro-oncology advancesArticle
- Proceedings of the Comprehensive Oncology Network Evaluating Rare CNS Tumors (NCI-CONNECT) Adult Medulloblastoma Workshop.Neuro-oncology advancesReview
- CERN 09-02: A phase 2 trial of carboplatin and bevacizumab for recurrent adult ependymomas.Neuro-oncology advancesArticle
- Characterization of recurrence patterns and outcomes of medulloblastoma in adults: The University of Texas MD Anderson Cancer Center experience.Neuro-oncology advancesArticle
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8 authors.
Funding
Abstract
purposePopulation-based cancer statistics, including histology-specific incidence, prevalence, and survival are essential to evaluating the total burden due to disease in a population. The National Cancer Institute's (NCI) Comprehensive Oncology Network Evaluating Rare CNS Tumors (NCI-CONNECT) was developed to better understand tumor biology and patient outcomes for 12 selected brain and other central nervous system (CNS) tumor histologies that are rare in adults to improve approaches to care and treatment. The aim of this study was to determine the incidence, prevalence, and survival of these selected rare histologies.
methodsData from the Central Brain Tumor Registry of the United States (CBTRUS) from 2000 to 2014 were used to calculate average annual age-adjusted incidence rates (AAIR) per 100,000 population overall and by sex, race, ethnicity, and age. NCI's Surveillance, Epidemiology and End Results (SEER) data were used to calculate relative survival (RS) estimates. Point prevalence for 2014 was estimated using annual age-specific incidence and survival from CBTRUS and SEER, respectively.
resultsOverall AAIR was 1.47 per 100,000 for all 12 rare histologies combined, with the highest histology-specific incidence in oligodendrogliomas (AAIR = 0.40/100,000). Overall, most histologies were more common in males, adults (age 40 + ), Whites, and non-Hispanics. Ependymomas were the most prevalent histology at 4.11 per 100,000; followed by oligodendrogliomas at 3.68 per 100,000. Relative survival at 1-, 5-, and 10-years was 82.3%, 64.0%, and 55.4%, respectively for all 12 selected brain and other CNS tumor types combined. Ependymomas had the highest RS (1-year = 94.2%, 5-year = 83.9%, 10-year = 78.6%) and gliosarcomas had the lowest relative survival rate (1-year = 42.5%, 5-year = 5.6%, 10-year = 2.9%) at all three time points.
conclusionsIncidence and prevalence of these rare brain and other CNS tumor histologies have not been previously reported. Along with survival, these data provide a statistical foundation to understand the impact of these cancers and provide important disease-specific data for the design of prospective clinical trials.
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